No abstract available.
Humans ; Lupus Erythematosus, Systemic* ; Renal Veins* ; Thrombosis*

The hyperimmunoglobulinemia E (Jobs) syndrome (HIES) is characterized by marked elevated levels of IgE, recurrent cutaneous and systemic staphylococcal infections, atopic-like dermatitis, and defective neutrophil chemotaxis. Three cases of HIES have been reported in Korea, but not in the dermatology literature. We report a case of HIES with cutaneous infections and MRSA (methicillin-resistant Staphylococcus aureus). A 15-month-old girl presented with intractable pruritic excoriated papular pustular skin lesions and multiple subcutaneous abscesses. Surgical drainage of the abscesses and a course of antibiotic treatment in addition to topical steroids for about 7 weeks resulted in a remarkable improvement.
Abscess ; Chemotaxis ; Dermatitis ; Dermatology ; Drainage ; Female ; Humans ; Hypergammaglobulinemia* ; Immunoglobulin E ; Infant ; Job Syndrome* ; Korea ; Methicillin-Resistant Staphylococcus aureus ; Neutrophils ; Skin ; Staphylococcal Infections ; Staphylococcus ; Steroids

One hundred seven isolates of Staphylococcus aureus from bovine mastitis were investigated for colony morphology in serum-soft agar (SSA), autoagglutination in salt, and capsular serotype. Capsular polysaccharide (CP) was purified and quantified from the extracts of clinical isolates. Overall, 89 isolates (83.2%) were diffuse in the SSA, without any difference in the proportion of diffuse colony between type 5 and type 8 strains. Some strains exhibited compact colonies in the SSA and expressed CP as determined by an enzyme-linked immunosorbent assay, indicating that compact morphology does not exclude encapsulation. The majority of the strains (11/12) showed autoagglutination in the salt aggregation test. The serotype 336 accounted for 46.7% of the isolates followed by serotype 5 (12.1%) and serotype 8 (12.1%). Particularly, twenty-six (24.3%) isolates reacted with two serotypes; 7 for type 8/336 and 19 for type 5/336. Five isolates (4.7%) were nontypeable with monoclonal antibodies specific for CP serotype 5, 8, or 336. The CP concentration in culture supernatants varied with the serotypes, and the total amount of CP produced by cells grown in a liquid medium was much less than that produced by cells grown on a solid medium. The Western blotting indicated that the CP bands of S. aureus serotype 5 and 8 were ranged in the molecular mass of 58-84 kilodalton (kDa), with additional bands in the region of approximately >or= 48 or Agglutination Tests ; Animals ; Bacterial Vaccines/administration & dosage ; Cattle ; Cattle Diseases/immunology/microbiology ; Female ; Mastitis, Bovine/*microbiology ; Polysaccharides, Bacterial/*classification/isolation & purification ; Serotyping/methods/veterinary ; Staphylococcal Infections/immunology/*veterinary ; Staphylococcus aureus/*classification/*immunology/isolation & purification

In order to investigate the efficacy and safety of oral fosinopril, a new phosphorus containing angiotensin converting enzyme inhibitor, a single dose of 10 to 20mg was administered in 23 hypertensive patients with diastotic blood pressure above 95mmHg and all other anti-hypertensive agents were not administered during 4 weeks of study. Blood pressure and heart rate were measured on the 2nd and 4th week of therapy. The complete blood count with platelet count, blood chemistry by SMA-12 and serum electrolytes were performed at the begining and 4th week of therapy. The urinalysis and electrocardiography were performed at the beginning and 4th week of therapy. Any kinds of side effects were actively questioned by the examining physicians. The following results were obtained : 1) At the beginning and 4th weeks of therapy, the average systolic and diastolic pressure were 170.0+/-17.6/101.6+/-6.1mmHg, 142.7+/-15.1/87.3+/-6.7mmHg respectively. The systolic and diastolic blood pressure were declined statistically significantly(p<0.05) throughout the period of treatment and diastolic blood pressure of all subjects except 3 patients(86%) was maintained below 90mmHg after 4th week of treatment. 2) There was no significant change in the pulse rate before and after therapy. 3) There were no significant changes in blood chemistry, serum electrolytes, hematologic findings, urinalysis and electrocardiographic findigns. 4) side effect were developed in 5 patients(23%) with dry cough, 3 patients(13%) with headache and 2 patients with facial edema but side effects were mostly mild in nature without potenitally serious episodes. These results suggested that antihypertensive therapy with onec-daily fosinopril was effective and well tolerated in essential hypertensive patients.
Antihypertensive Agents ; Blood Cell Count ; Blood Pressure ; Chemistry ; Cough ; Edema ; Electrocardiography ; Electrolytes ; Fosinopril* ; Headache ; Heart Rate ; Humans ; Hypertension ; Peptidyl-Dipeptidase A ; Phosphorus ; Platelet Count ; Urinalysis

We report a case of neuroblastoma with multiple skin metastases as a chief complaint in a 2-month-old girl. the skin lesions were rnultiple, pea-sized, bluish, nontender, moable subcutaneous nodules on abdomen, back and scalp. Histopathology showed small round or poly gonal tumor cells which have deeply stained, basophilic, hyperchromatic nuclei with some mitoses. Th.se tumor cells showed clumping tendency which is one of early menifestations of rosette formation. Immunohistochemically positive reaction was demonstrated by anti-NSE(neuron specific enolase) antilody but negative reaction by anti-NFP (neurofilament proteiin ) antibody. She has been succesfully treated with combined chemotherapy for 10 months without relapse.
Abdomen ; Basophils ; Drug Therapy ; Female ; Humans ; Infant ; Mitosis ; Neoplasm Metastasis* ; Neuroblastoma* ; Phosphopyruvate Hydratase ; Recurrence ; Rosette Formation ; Scalp ; Skin*

BACKGROUND: The monitoring of end-tidal CO2 tension (PETCO2) during high frequency jet ventilation (HFJV) has been unsatisfactory because of a small tidal volume and slow response time of CO2 analyser, although several authors have reported strategies of successful PETCO2 measurement during HFJV. The aim of this study was to assess the validity of tracheal CO2 tension (PtCO2) as a PaCO2 during HFJV. METHODS: We studied 24 patients undergoing laryngomicrosurgery during HFJV (rates: 100/min; I:E= 0.2; driving pressure: 0.25-0.35 MPa) through a 12 Fr. polyethylene injector placed 6-7 cm below the vocal cord. A gas sampling line was placed longitudinally against the injector and they were wrapped with aluminum foil. Continuous capnography was recorded during 20 minutes of HFJV. Every 5 minutes of HFJV, PtCO2 was obtained from the plateau value of CO2 wave after the stopping of JV and arterial blood gas analysis was done at 20 minutes of HFJV comparing PaCO2 to PtCO2. A Pearson's product moment correlation and regression analysis between PtCO2 and PaCO2 and the agreement between the two methods using Bland-Altman method were assessed. RESULTS: A regression analysis (R2=0.928) and a Pearson's product moment correlation (r=0.965, P<0.001) indicated a strong correlation of PtCO2 and PaCO2 during HFJV. The difference against a mean scatter diagram showed a relative good agreement between the two method (mean difference: 1.58 (SD 2.22) mmHg; limit of agreement: 2.86 and -6.02). CONCLUSIONS: PtCO2 obtained from a plateau of CO2 wave on capnography after interruption of HFJV can accurately reflect PaCO2 during HFJV in relative.
Aluminum ; Blood Gas Analysis ; Blood Gas Monitoring, Transcutaneous ; Capnography ; High-Frequency Jet Ventilation* ; Humans ; Polyethylene ; Reaction Time ; Tidal Volume ; Vocal Cords

Diabetic nephropathy is a common complication of diabetes mellitus and is the leading cause of chronic kidney disease. Glycemic and blood pressure control constitute the main strategies of diabetic nephropathy prevention and treatment. However, despite current therapies, nephropathy progresses to renal failure and end-stage renal disease in many patients. Therefore, new therapeutic strategies targeting different pathophysiological mechanisms are needed. This review article briefly summarizes the standard therapy for diabetic nephropathy and also describes recent advances in potential renoprotective agents that could be used to prevent the development or progression of diabetic nephropathy.
Blood Pressure ; Diabetes Mellitus ; Diabetic Nephropathies* ; Humans ; Kidney Failure, Chronic ; Renal Insufficiency ; Renal Insufficiency, Chronic

PURPOSE: Papillary thyroid microcarcinomas (PTMCs) have the same histological features as papillary thyroid cancer, but they are 1.0 cm or less in diameter. They can metastasize to the regional lymph nodes and distant sites, but its ability to cause significant morbidity and mortality has been questioned. Because of this reason, the extent of thyroid tumor resection remains an issue of controversy. This study is aimed at identifying the statistically significant factors that are associated with recurrence and we also wanted to devise an appropriate surgical treatment plan for PTMC patients. METHODS: The retrospective review (350 cases, 1990.1~2004. 11) was obtained from Seoul National University Hospital (SNUH). The mean age at the time of diagnosis was 46.5± 11.0 (yrs) (range: 12~75). The mean overall length of follow- up was 37.70± 36.03 months (range: 1~169). The PTMCs were treated with total and subtotal thyroidectomy or lobectomy. The invasiveness and lymph node metastasis (LNM) from 350 PTMCs were analyzed according to the size, multiplicity, bilaterality of the tumor and the perithyroidal invasion. Fishers exact test and the exact logistic regression test were used for the stratified analysis. RESULTS: 350 of the 2187 papillary carcinoma were PTMCs. There were 296 females (84.6%) and 54 males (15.4%) in the study. Invasion into the perithyroidal tissue was common (128/336, 38.1%). There were 68 patients with LNM among the 312 total patients (21.7%). The group with either perithyroidal invasion or LNM showed a significantly higher recurrence rate than those group having neither one (4.8% vs. 10.9%, 4.5% vs. 19.1%, respectively). Even for tumor smaller than 1 cm, a larger-sized tumor resulted in a poorer prognosis. CONCLUSION: PTMC is an early stage carcinoma with the capability of tissue invasion, lymph node metastasis and multiplicity. Based on this study, total thyroidectomy is recommended for significant portion of the PTMCs. Furthermore, more careful imaging studies (such as neck ultrasonography or neck CT scan) are needed to detect contralateral lesions or neck lymph node metastasis.
Carcinoma, Papillary ; Diagnosis ; Female ; Humans ; Logistic Models ; Lymph Nodes ; Male ; Mortality ; Neck ; Neoplasm Metastasis ; Prognosis ; Recurrence ; Retrospective Studies ; Seoul ; Thyroid Gland* ; Thyroid Neoplasms ; Thyroidectomy ; Ultrasonography

OBJECTIVE: We have previously shown that DICAM inhibits LPS-mediated macrophage differentiation. However, less is known about the exact action mechanisms of DICAM on the macrophage function and differentiation. METHODS: To induce differentiation into a resting M0 macrophage, THP-1 cells were cultured with 100 nM PMA for 24 h, and then rested for 3 days. THP-1 cells were infected with 50 moi of control LacZ- or DICAM-containing adenovirus. The RNA expression profile associated with DICAM during THP-1 differentiation was analyzed with a microarray chip and in silico analysis with Ingenuity Pathway Analysis (IPA) program. RESULTS: A disease and function analysis of the microarray data in DICAM-overexpressed THP-1 cells revealed a suppression in the expression of multiple genes involved in the response of myeloid cells and phagocytes, and an increase of genes associated with apoptosis of fibroblast cell-line, and viral infection and replication. The canonical pathway analysis also showed the most prominent changes of signaling pathways that involve inflammation responses. An upstream regulator analysis identifyingmolecules upstream of the genes that potentially explain the observed expression changes revealed that IRF7 and the genes in type 1 interferon system, such as IFNA2 and IFNAR,was significantly attenuated by DICAM. A mechanistic network analysis confirmed a direct causal association between IRF7 and type 1 interferon system. A real-time RT-PCR analysis validating the microarray data verified the significant suppression of IRFs, IFNA2, and IFNB1. CONCLUSION: These results suggest that DICAM can be a critical regulator of type 1 interferon system, which is an essential mediator in the process of intracellular infection and systemic lupus erythematosus.
Adenoviridae ; Apoptosis ; Computer Simulation ; Fibroblasts ; Inflammation ; Interferons* ; Lupus Erythematosus, Systemic ; Macrophages* ; Myeloid Cells ; Phagocytes ; RNA

BACKGROUND: Psoriasis is belived to be a disorder of keratinocyte hyperproliferation mediated by T helper cells. Cyclosproine is one of the immunosuppressants and there have been several studies showing the benefcial effects of cyclosporine on psoriasis. OBJECTIVE: To evaluate the clinical efficacy, tolerability, and adverse effects of cyclosporine, a randomized open uncontrolled multicenter study was conducted in 15 university hospitals in Korea. METHOD: There were 68 total trial cases and among them 16 patients dropped out from the study. The reported reasons for stopping the use of the medication under study prematurely were lack of cooperation(seven cases), adverse effects(six cases), the ineffectiveness of the medication(two cases), and another(one case). The drug was administered for 18 weeks to 52 patients. The initial dosage of the drug was 2.5mg/kg/day which was maintained or increased to 4mg/kg/day or 5mg/kg/day according to the PASI score reduction rate at the 6th and 12th week. The PASI score was measured and Iaboratory tests and observation of adverse events were done. RESULTS: At the end of therapy PASI score reduction rate of more than 66% occured in 40 patients (76.9%). The PASI score was significantly reduced from 20.0 to 5.4 after treatment for 18 weeks. The social disability score was significantly decreased. Pruritus and nail involvement were also significantly decreased. The change of systolic and diastolic blood pressure were statistically not significant. The 30% increase of serum creatinine level compared to the baseline was observed in six patients(11.5%) at the 6th week, three patients(5.8%) at the 12th week, five patients(9.6%) at the 18th week, but no patients showed an increase above the normal range.The increase of serum total bilirubin and SGOT was observed in six patients(11.5%) and one patient(1.9%0, respectively. The clinical adverse events reported during the study were gastrointestinal trouble(seven cases, 13.5%), hypertrichosis(two cases, 3.8%), generalized weakness(two cases, 3.8%0, paresthesia(one case, 1.9%), hypertension(one case, 1.9%), disturbance of erection(one case, 1.9%). The overall assessment of efficacy and tolerability by investigator and patients were mostly good or very good. CONCLUSION: Generally cyclosporine was well accepted and tolerated and low dose cyclosporine therapy-2.5mg/kg/day to 5mg/kg/day-is an effective therapeutic modality for the treatment of psoriasis.
Aspartate Aminotransferases ; Bilirubin ; Blood Pressure ; Creatinine ; Cyclosporine* ; Hospitals, University ; Humans ; Immunosuppressive Agents ; Keratinocytes ; Korea ; Pruritus ; Psoriasis* ; Research Personnel ; T-Lymphocytes, Helper-Inducer